Primary intrahepatic malignant mesothelioma (PIHMM) is an extremely rare tumor with clinicopathological characteristics that remain to be elucidated. The current study presents the case of a 68-year-old female with PIHMM and multiple lymphadenopathies due to non-tuberculous mycobacteria. The patient presented with an intrahepatic tumor, 70 mm in diameter, in the right lobe of the liver. An ultrasound-guided fine-needle aspiration biopsy of the liver tumor revealed findings that were consistent with an intrahepatic malignant mesothelioma. The systemic lymph node swellings were due to epithelioid granulomas that were caused by non-tuberculous mycobacteria. However, a hepatic rupture occurred due to the rapid growth of the liver tumor and consequently, a surgical resection was not performed. A review of the literature revealed that the clinicopathological characteristics of PIHMM are similar to those of non-occupational mesothelioma. However, PIHMM is usually a solitary tumor and is rarely associated with cavity effusion in contrast with conventional mesothelioma. Therefore, surgical resection with curative intent is often recommended for patients with PIHMM.